Case presentation
A 37-year-old female patient with petechia and purpura from 7 months ago was diagnosed with leukocytoclastic vasculitis confirmed by biopsy. Despite receiving anti-inflammatory treatments, she was admitted to our hospital due to the gradual progression of constitutional symptoms, pancytopenia, and new pulmonary and renal involvement. Diffuse petechia and purpuric lesions were detected, especially in the lower limb. In Table 1, initial lab data results are shown.
Splenomegaly was detected in the abdominal ultrasound. Computed tomography (CT) of the chest showed consolidations in the left upper lobe. Bone marrow aspiration and biopsy showed 40% cellularity with increased erythroid precursors. Megaloblastic changes in both erythroid and myeloid precursors were detected as well. According to these findings, myelodysplastic syndrome secondary to leukocytoclastic vasculitis was the main reason for megaloblastic changes. Megaloblastic anemia was also considered a second differential diagnosis. Due to the history of VSD in childhood, a tans thoracic echocardiogram was done. Multiple mobile masses in RVOT and highly mobile masses at the atrial side of the tricuspid valve and the ventricular side of the pulmonary valve were seen (Figure 1, video). A turbulent flow in RVOT with a peak gradient of about 80 mmHg was detected at the hypertrophied muscle bundle site (Figure 1, video). A transesophageal echocardiogram for further evaluation was performed. The muscle bundle in the right ventricle (RV) was divided it into two chambers: A proximal chamber on the side of the RV inflow and a distal chamber on the side of the pulmonary valve. Many masses were attached to both the muscle bundle and along the distal chamber involving RV endothelium and pulmonary valve (Figure, video). Multiple masses and destruction of the tricuspid valve leaflets resulted in severe regurgitation (Figure, video).
The patient was diagnosed with DCRV and IE. After consultation with the cardiac surgeon and infectious disease specialist, empiric antibiotics therapy was initiated, and the patient underwent cardiac surgery. Pulmonary and tricuspid valve replacement with bioprosthesis valves was performed. Although VSD was not found in the echocardiogram, probably due to high RV pressure, surgery proved it and was repaired with a pericardial patch. The patient’s condition improved after surgery and antibiotics therapy, and she was discharged with normal lab results. The patient was in good clinical and echocardiographic condition and free of any major event in the one-year follow-up.